Ectrodactyly
From Wikipedia, the free encyclopedia
Ectrodactyly, commonly known as Lobster claw syndrome, is a rare congenital deformity of the hand where the middle digit is missing, and the hand is cleft where the metacarpal of the finger should be. This split gives the hands the appearance of lobster claws.
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[edit] Features
- Ectrodactyly is an inherited condition, and often occurs in both the hands and the feet. Its inheritance pattern is autosomal dominant.
- It affects about 1 in 90,000 babies, with males and females equally as likely to be affected.
- It is treated surgically to improve function and appearance. Prosthetics may also be used, and genetic counselling given to parents with the condition.
[edit] Other names
- Lobster claw hand
- Split hand deformity
- Split hand/foot malformation (SHFM)
- Cleft hand
- Ectrodactilia of the hand
- Karsch-Neugebauer syndrome
[edit] Causes
There are different forms of the disorder and each of them are connected with a different genetic mutation. Type I, the most frequent form has been found to be a mutation on chromosome 7 in a region that contains two homeobox genes, DLX5 and DLX6.[1]
[edit] Famous people with ectrodactyly
- Vadoma
- Bree Walker - News anchor for 20 years KCBS news in Los Angeles; actress on HBO drama Carnivale. She also appears in the fourth season premiere of FX's Nip/Tuck playing a real estate agent counseling parents expecting a child with ectrodactyly.
- Grady Stiles
[edit] Trivia
- Covenant Elites from Halo: Combat Evolved and Halo 2, although perfectly normal, have hands that look like they have Ectrodactyly because the first two digits split in the middle.
[edit] References
- Causative gene for human "lobster claw" syndrome identified
- Embracing her 'inner freak' USA Today interview with Bree Walker